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Received: 7 October 2013 / Accepted: 10 December 2013 / Published online: 14 February 2014© Springer-Verlag Berlin Heidelberg 2013

Cerebrovascular Complications in a Patient with Hereditary Hemorrhagic Telangiectasia (HHT, Rendu–Osler Disease)

M. Meier · S. Greschus · H. H. Schild · D. R. Hadizadeh

Clin Neuroradiol (2015) 25:89–92DOI 10.1007/s00062-013-0277-z

the patient developed new symptoms of vertigo and a right-sided scotoma within 5 days due to an infarction of the left peripheral posterial cerebral artery (PCA) territory. Shortly after her release from the hospital, the patient was readmit-ted with paresis of the left arm. Both computed tomography (CT) and MRI showed a recurrent abscess and an infarction of the left PCA territory (Fig. 2).

After subsequent resurgery and intravenous antibiosis, the paresis of the left arm improved. However, 2 weeks later, the patient presented with seizure and sensory aphasia. Besides a residual abscess in the resection cavity, MRI showed a new hemorrhagic infarction in the left temporal lobe and sub-arachnoidal hemorrhage, as well as thrombosis of the left Labbé vein and the left transverse and sigmoid sinus (Fig. 3).

CT of the thorax revealed a 3.5-cm-measuring AVM in the left upper lobe as well as various smaller AVMs in both lungs. As the most probable origin of embolization, the AVM was occluded using an Amplatzer vascular plug (AVP) and coils in subsequent catheter angiography (Fig. 4).

MRI of the brain 2 weeks later showed regressive edema in the region of surgery and around the hemorrhagic infarc-tion. With mild aphasia, impaired short-term memory, sco-toma, and a decrease in fine motor skills, but no paresis, the patient was released into rehabilitation in an otherwise good general condition.

Discussion

Most of the complications of HHT refer to pulmonary AVMs, which occur in up to 33 % of patients [1]. There-fore, an early diagnosis of pulmonary arteriovenous mal-formations (PAVMs) is essential to protect these patients from severe complications. PAVMs usually become clini-cally apparent due to hypoxemia, with dyspnea on exertion

Introduction

Hereditary hemorrhagic telangiectasia (HHT) is an autoso-mal dominant genetic disorder characterized by recurrent epistaxis, arteriovenous malformations (AVMs), mucocu-taneous telangiectasia, and family history for the disease [1]. Its prevalence shows regional diversity, ranging from 1/5,000 to 1/10,000 [1].

Patients with HHT suffer from a particularly higher risk of brain abscesses as a direct consequence of right-to-left shunting due to AVMs [1], while usually a brain abscess develops secondary to other infectious foci, either hema-togenously or locally spreading [2]. Besides, AVMs also predispose for arterial embolic thrombosis with subsequent cerebral infarction.

Case Report

A 51-year-old female patient with known HHT was admit-ted to the emergency department with reduced vigilance, fever (39 °C), and paresis of the left arm. Magnetic reso-nance imaging (MRI) revealed a cerebral abscess, leading to immediate admission into the department of neurosurgery (Fig. 1).

The abscess was drained via borehole trepanation. Neither an otorhinolaryngological referral nor echocardiography exposed a possible infectious focus. Intravenous antibiotics were given for 2 weeks. The paresis distinctly improved, but

M. Meier () · S. Greschus · H. H. Schild · D. R. HadizadehDepartment of Radiology, University of Bonn,Sigmund-Freud-Str 25, 53105 Bonn, Germanye-mail: michael.meier@ukb.uni-bonn.de

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being the most common symptom [1]. Yet, they may remain asymptomatic unless complications occur [1, 3].

Emboli are related to right-to-left shunting, which can lead to brain abscess and arterial thrombosis with subse-

quent cerebral infarction [1, 3]. Both occur in up to 19 % of HHT patients with PAVMs [1]. It has not been fully understood whether emboli arise from underlying venous thrombosis or whether they form inside the aneurismal por-

Fig. 2 Right frontal ring-en-hancing lesions (arrow in a) with perifocal edema (dotted arrows in a) in contrast-enhanced computed tomography. Enhancement of the adjacent meninges (arrows in b, coronal contrast-enhanced T1-weighted spin-echo) and early-stage infarction in the medial part of the posterial cerebral artery territory (arrows in c, transver-sal fluid-attenuated inversion recovery) in magnetic resonance angiography

Fig. 1 Nodular, ring-enhancing lesion (arrow in a, transversal contrast-enhanced T1-weighted spin-echo) with perifocal edema (arrows in b, transversal fluid-attenuated inversion recovery) and restricted diffusion [arrow in c and d, diffusion-weighted imaging (b = 1,000); Apparent Diffusion Coefficient map]

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most neoplastic lesions contain less fluid, and subsequently less necrotic tissue and inflammatory cells [8].

Close cooperation of the departments of neuroradiology and neurosurgery is mandatory regarding preoperative plan-ning and follow-up imaging. Therapy includes resection of the abscess, drainage of pus, and intravenous antibiosis based on antibiogram [2]. The overall prognosis varies sig-nificantly, with a lethality rate of 10–40 % [2].

The underlying HHT can only be treated symptom-atically, as its progression and the reoccurrence of com-plications cannot be fully prevented. When PAVMs are diagnosed, guidelines recommend transcatheter emboliza-tion of feeding arteries with coils or AVPs [3]. Emboliza-tion is an effective procedure with a low complication rate that can reduce the incidence of brain abscess and stroke [3, 4, 9]. Coils are usually preferred in embolization of small vessels [9], whereas immediate occlusion of bigger ves-sels can be achieved with AVPs [4]. The recanalization rate of coils is 8–15 %; the risk of incomplete occlusion using AVPs appears to be lower [9]. Surgical therapy is only rec-ommended in cases of serious complications and in cases where embolization is not available [1].

A predisposition to venous thrombosis in patients with HHT is not known, yet to our knowledge, there is one case report referring to it [10]. In this particular case, the patient also suffered from moderate hyperfibrinogenemia that may also have contributed to venous thrombosis. However,

tion of the fistula [4]. The standard of reference for detec-tion of PAVMs is CT [1]. However, other possible reasons for emboli, including endocarditis, local infections, atrial thrombi, and atherosclerotic lesions, should be excluded in the first place.

Besides general symptoms, brain abscesses often become symptomatic by cephalgia or vomiting, or focal symptoms like paresis or vision disorders due to their mass effect [2]. In an acute setting, CT is the preferred tool to detect mass lesions, edema, and possible complications that may then be further evaluated using MRI.

A brain abscess begins as focal cerebritis with only dis-crete signal alterations in CT and MRI [2, 5]. It then develops into a collection of pus surrounded by a well-vascularized capsule [2], visualized by CT as a hypodense, ring-enhanc-ing lesion [5] or by MRI as a round lesion with hyperintense signal in T2-weighted images and hypointense signal with marked rim enhancement in T1-weighted images [5, 6].

The most important differential diagnosis of a brain abscess is malignancy. Both entities show mass effects and perifocal edema as well as ring enhancement, yet in many cases, they can be distinguished with diffusion-weighted imaging (DWI) [6–8]. Being a viscous fluid consisting of inflammatory cells, necrotic tissue, and bacteria, pus restricts free motion of water molecules and therefore shows high signal in DWI [7]. In contrast, the necrotic portion of

Fig. 4 Transversal thick-slice maximum intensity projection of com-puted tomography shows a 3.5-cm-large arteriovenous malformation in the upper lobe of the left lung (arrow in a); treatment of the lesion

(arrow in b, digital subtraction angiography) by interventional place-ment of an Amplatzer vascular plug and coils (arrow in c, digital sub-traction angiography)

Fig. 3 Residual abscess (dotted arrow in a) with perifocal edema (arrows in a) in transversal fluid-attenuated inversion recovery and hemorrhage in the left temporal lobe (arrow in b, transversal contrast-enhanced T1-weighted spin-echo) as well as thrombosis of the left transverse and sigmoid sinus (arrows in c, frontal view of maximum intensity projection of 3D phase contrast angiography) and the Labbé vein (dotted arrow in c)

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4. Hinterseer M, Becker A, Barth AS, Kozlik-Feldmann R, Winter-sperger BJ, Behr J. Interventional embolization of a giant pulmo-nary arteriovenous malformation with right-left-shunt associated with hereditary hemorrhagic telangiectasia. Clin Res Cardiol. 2006 Mar;95(3):174–8.

5. Horger M, Nägele T, Joanoviciu SD, Danz S, Ernemann U. Cerebritis and brain abscess: imaging findings. Rofo. 2009 Sep;181(9):831–5.

6. Chang SC, Lai PH, Chen WL, Weng HH, Ho JT, Wang JS, Chang CY, Pan HB, Yang CF. Diffusion-weighted MRI features of brain abscess and cystic or necrotic brain tumors: comparison with con-ventional MRI. Clin Imaging. 2002 Jul-Aug;26(4):227–36.

7. Lai PH, Ho JT, Chen WL, Hsu SS, Wang JS, Pan HB, Yang CF. Brain abscess and necrotic brain tumor: discrimination with pro-ton MR spectroscopy and diffusion-weighted imaging. AJNR Am J Neuroradiol. 2002 Sep;23(8):1369–77.

8. Ebisu T, Tanaka C, Umeda M, Kitamura M, Naruse S, Higuchi T, et al. Discrimination of brain abscess from necrotic or cystic tumors by diffusion-weighted echo planar imaging. Magn Reson Imaging. 1996;14(9):1113–6.

9. Andersen PE, Kjeldsen AD. Interventional treatment of pulmo-nary arteriovenous malformations. World J Radiol. 2010 Sep 28;2(9):339–44.

10. Sturm W, Gaenzer H, Heininger-Rothbucher D, Neumayr G, Kirchmair R, Patsch J, Weiss G. Unsuspected mesenteric vein thrombosis in a patient with a hereditary bleeding disorder. Blood Coagul Fibrinolysis. 2003 Sep;14(6):599–600.

alterations of blood flow, which commonly occur in patients with HHT, should be considered as possible risk factors and should  raise awareness of an increased risk of venous thrombosis. In our case, the thrombosis was most likely due to cerebral inflammation—a correlation that physicians should also be aware of.

Conflict of Interest On behalf of all authors, the corresponding au-thor states that there is no conflict of interest.

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